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Assay
99% (TLC)
form
powder
packaging
package of 1 × 500 μg (860952P-500UG)
manufacturer/tradename
Avanti Polar Lipids
lipid type
sphingolipids
shipped in
dry ice
storage temp.
−20°C
SMILES string
O[C@H]1[C@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@@H]([C@H](O)[C@H]2O)O[C@H]3O[C@H](CO)[C@@H]([C@H](O)[C@H]3O)O)[C@@H]1O
General description
Globotriaosylsphingosine (Lyso GB3) is a deacylated form of globotriaosylceramide. It is a circulating bioactive glycolipid.
Application
Globotriaosylsphingosine (Lyso GB3) has been used as a standard in generating calibration curve via ultra-performance liquid chromatography (UPLC) method.
Biochem/physiol Actions
Globotriaosylsphingosine (Lyso GB3) is a potential biomarker of Fabry disease.
Packaging
2 mL Amber Serum Vial with Stopper and Crimp Cap (860952P-500UG)
WGK
WGK 3
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Regulatory Information
监管及禁止进口产品
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Find documentation for the products that you have recently purchased in the Document Library.
The Fabry disease-associated lipid Lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain
Choi L, et al.
Neuroscience Letters, 594, 163-168 (2015)
Lyso-Gb3 activates Notch1 in human podocytes
Sanchez N, et al.
Human Molecular Genetics, 24(20), 5720-5732 (2015)
Rui Quinta et al.
Gene, 536(1), 97-104 (2013-12-18)
Fabry disease is an X-linked lysosomal storage disease (LSD) caused by deficient activity of α-Galactosidase A (α-Gal A). As a result, glycosphingolipids, mainly globotriaosylceramide (Gb3), progressively accumulate in body fluids and tissues. Studies aiming at the identification of secondary lipid
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